Miniati Douglas N, Chintagumpala Murali, Langston Claire, Dishop Megan K, Olutoye Oluyinka O, Nuchtern Jed G, Cass Darrell L
Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, TX 77030, USA.
J Pediatr Surg. 2006 Jan;41(1):66-71. doi: 10.1016/j.jpedsurg.2005.10.074.
BACKGROUND/PURPOSE: Pleuropulmonary blastoma (PPB) is a rare primary neoplasm of pleuropulmonary mesenchyme. Fewer than 170 children have been reported, and few single institutions have reported more than several cases. Treatment for this condition is primarily surgical resection; however, increasing experience suggests that adjuvant chemotherapy may decrease recurrence and improve outcome.
We reviewed the charts of all children with PPB treated at our institution since 1960. We reviewed the prenatal features, clinical presentation, operation, pathological findings, adjuvant treatment, and outcome.
Ten children (6 boys and 4 girls) were treated for PPB at a mean age of 3.2 +/- 4.3 years. In 2, a cystic lung mass was diagnosed prenatally, and in 8, a cystic or solid and cystic lung mass was diagnosed postnatally (right lung, 3; left lung, 4; and bilateral, 3). In no patient was PPB considered preoperatively. Surgical resection was performed at 1 day to 11 years (median, 23 months) of age. Seven children had complete resection; 1 had microscopic residual disease, and 2 had gross residual disease. Pathology showed type I PPB in 7, type II in 1, and type III in 2. Five patients received adjuvant chemotherapy with vincristine, actinomycin, and cyclophosphamide-based regimens. At follow-up (mean, 7.7 +/- 11.5 years; range, 1-456 months), children with type I PPB have no evidence of disease (n = 6) or are lost to follow-up (n = 1), whereas all those with type II/III PPB have died of the disease.
PPB must be included in the differential diagnosis of a fetus, neonate, or child with a cystic lung mass. This finding supports early resection of these lesions rather than observation or treatment with nonoperative strategies.
背景/目的：肺胚细胞瘤（PPB）是一种罕见的肺胸膜间充质原发性肿瘤。据报道，患病儿童不足170例，很少有单一机构报告过数例以上的病例。这种疾病的治疗主要是手术切除；然而，越来越多的经验表明，辅助化疗可能会降低复发率并改善预后。
我们回顾了自1960年以来在本机构接受治疗的所有PPB患儿的病历。我们回顾了产前特征、临床表现、手术、病理结果、辅助治疗和预后情况。
10名儿童（6名男孩和4名女孩）接受了PPB治疗，平均年龄为3.2±4.3岁。2例在产前诊断为肺囊性肿块，8例在出生后诊断为囊性或实性及囊性肺肿块（右肺3例；左肺4例；双侧3例）。术前均未考虑为PPB。手术切除在1天至11岁（中位年龄23个月）时进行。7名儿童完全切除；1名有微小残留病灶，2名有肉眼可见的残留病灶。病理显示7例为I型PPB，1例为II型，2例为III型。5例患者接受了以长春新碱、放线菌素和环磷酰胺为基础方案的辅助化疗。在随访中（平均7.7±11.5年；范围1 - 456个月），I型PPB患儿无疾病证据（n = 6）或失访（n = 1），而所有II/III型PPB患儿均死于该疾病。
对于患有肺囊性肿块的胎儿、新生儿或儿童，必须将PPB纳入鉴别诊断。这一发现支持早期切除这些病变，而不是观察或采用非手术策略进行治疗。
